Primary Ewing's sarcoma of the cranium

OBJECTIVE: We analyzed the data for a series of 14 patients with primary Ew ing's sarcomas of the cranium who were treated since 1985. Our aim was to a ssess the long-term outcomes and the selection of appropriate treatment met hods. METHODS: The patients were reviewed with respect to their clinical presenta tions, treatment, and outcomes. Computed tomographic scanning of the brain was performed for all patients. Skeletal surveys with routine radiographs a nd technetium-99 bone scans to detect extracranial Ewing's sarcomas were pe rformed for all patients. For ail 14 patients, radical tumor excision was a chieved surgically. Ail patients were then subjected to adjuvant multidrug chemotherapy and radiotherapy. The follow-up periods ranged from 8 months t o 8 years (mean, 4.25 yr). RESULTS: The predominant presenting features were headaches, increased intr acranial pressure, and scalp swelling. Excision was nearly total for nine p atients and total for five patients. All patients experienced uneventful po stoperative courses. One patient experienced a local recurrence, which was detected 2 years after surgery. This recurrent tumor was completely excised , and additional chemotherapy was administered. Eight of the 14 patients (5 7.1%) studied have survived 5 years or longer. CONCLUSION: Although primary Ewing's sarcoma of the cranium is a malignant bone tumor, it is associated with a good prognosis when treated with radica l surgery, aggressive multidrug chemotherapy, and radiotherapy.